Paediatric Congenital Heart Defects Case Presentation

1. WELCOME TO YOUR
WORST NIGHTMARE.
The collapsed neonate.

2. SUMMARY
 Case
 Discussion
• Congenital heart disease in neonates presenting to ED

3. THE CASE
 12 week old girl, ex 32 week prem (4 weeks corrected)
• 2 days increased WOB
• Poor feeding
 Mother Burmese and speaks no english

4. THE CASE
 Initially triage 3, nurse asks for review as becoming floppy and
increased work of breathing
• Afebrile, RR 60, sats 91% RA, P 170, unable to obtain BP
• CRT 6 seconds, cool peripheries
• Floppy, grunting, no spontaneous cry, is spontaneously moving
• Asymmetry of chest
• Moderate recessions, bilateral crackles
• Grade 3 on 4 systolic murmur, loudest left sternal edge

5. ADDITIONAL
EXAMINATION FINDINGS
 ????

6. EXAM
 Trachea midline
 Equal air entry and percussion
 ? Trans-illumination (not performed)
 Hepatomegaly
 Femoral pulses not able to be palpated (however nil could be palpated)
 No signs of trauma

7. DDX
 ???

8. INITIAL INVESTIGATIONS
 ??

9. VBG (FROM HELL!)
 pH 6.70
 pCO2 58
 HCO3 7
 Na 138
 K 7.5
 Chl 114
 BSL 2.6
 Lactate 20
 Hb 78

10. CHEST XRAY

11. MANAGEMENT
 ???

12. MANAGEMENT
 A and B and C
• Neopuff assisted ventilation
• Total 20ml/kg fluid boluses
• Manage hyperkalaemia
• Calcium gluconate
• HCO3
• Salbutamol nebs
• adrenaline bolus then infusion
• Intubation
• Rocuronium 1.2 mg/kg, fentanyl 1 mcg/kg
• Size 3.5 ETT, hyperventilate
• Prostin infusion

13. MANAGEMENT
 D
• 3ml/kg 10% dextrose then infusion
• Morphine and midazolam infusion
 Other
• Ceftriaxone
• Get help

14. HELP!
 Cardiology
• ECHO = abnormal mitral valve, severe MR, severely dilated LA,
dilated and hyperdynamic LV
 PICU
• Transferred to PICU within 90 minutes of presentation

15. FINAL DIAGNOSIS
 Congenital MR
• Shock, CCF
• Multiorgan failure – liver, renal, thyroid, coagulopathic
 Collapse precipitated by inter-current illness
• Rhinovirus

16. CHD in the ED
 A practical classification
 Key presenting features
 Management principles
 Not a comprehensive review. Not discussing
 Individual lesions
 Murmurs
 Tet spells
 Arrhythmias

17. How they present
 Congestive heart failure
 Left to right shunts
 LVOTO
 Cyanosis (not responsive to oxygen)
 Right to left shunts
 Shock/collapse
 Obstructive left heart lesions
 Incidental heart mummur
 Arrhythmia

18. Fetal Circulation

19. CCF
Pathophysiology
 Left to right shunt = volume overload of the right heart and lungs
 Overtime – PVR decreases, right heart and lung flow increases
 Is responsive to oxygen
 left sided obstructive lesions
 Pressure overload left heart, back-pressure to lungs
Differential diagnosis
 ASD
 VSD
 PDA
 Endocardial cushion defects
 (left sided obstructive lesions)

20. CCF
 Timing
 First few months of life
 Symptoms
 FTT, poor weight gain
 Sweating, respiratory distress, esp with feeds
 Signs
 Sweating, wheeze, gallop, hepatomegaly, peripheral oedema
(rarely)
 May be cyanotic but oxygen responsive (Improves with crying)
 Chest x-ray
 Congestion, effusions

21. Cyanosis
 Pathophysiology
 Right to left shunt – mixing
 Flow through the lungs is either increased (eg Transposition) or decreased (eg
TOF)
 Pulmonary blood flow is duct dependent
 Is unresponsive to oxygen
 Differential diagnosis
 1T
 2T
 3T
 4T – 10% of all CHD, number 1 cause beyond infancy
 5T
 (PS)

22. Cyanosis
 Timing
 Variable
 Symptoms
 Mild, minimal respiratory distress
 Signs
 “comfortably blue”
 Central cyanosis (tongue and mucous membrane of mouth, not lips),worsens with
crying
 Hyperoxia test
 high flow O2 for 10 minutes, if sats increase > 10%, likely pulmonary cause
 Sats probe right arm (pre-ductal)
 Chest x-ray
 Variable pulmonary markings, dependent on lesion
 cardiomegaly

23. Shock
 Pathopysiology
 Left ventricular outflow tract obstruction
 Either level of heart or aorta
 Systemic blood flow is duct dependent
 Rarely severe regurgitant lesions
 May cause back pressure = CCF
 Differential diagnosis
 Hypoplastic left heart
 Critical aortic stenosis
 Interrupted aortic arch
 Coarctation

24. Shock
 Timing
 Usually within first few days of life, up to 3 weeks
 Symptoms
 Irritability, poor feeding, respiratory distress
 Signs
 Unwell, collapsed, hypotension
 Cyanosis and CCF (50%)
 Murmur (50%)
 Weak femoral pulses
 Chest x ray
 Cardiomegaly (85%)

25. Chest X-RAY
 3 key features
 Size of heart
 Shape of heart and mediastinum
 Degree of pulmonary vascular markings

29. Management Principles
 Oxygen, ABCs
 NS boluses if shocked
 10ml/kg
 Get help early
 Cardiology,+/- NICU/PICU
 Prostaglandin if shock or decompensated cyanotic heart disease
 Consider differentials
 Sepsis – Abs
 Respiratory
 Metabolic – check a BSL
 NAI

30. Prostaglandin E1
 Potent vasodilator, opens the the ductus arteriosis
 Majority of cardiac lesions occuring in the 1st 3 weeks of life presenting with
shock or cyanosis are duct dependent – should start empirically in
conjunction with Cardiology and NICU/PICU
 Remember,
 Preserves pulmonary flow in cyanotic lesions
 Preserves systemic flow in obstructive lesions
 Works within 15 minutes – either improves shock or cyanosis
 Infusion start @ 0.05 to 0.1 mcg/kg/min, up to 0.4 mcg/kg/min
 Adverse effects – apnoea (may need intubation)

31. Take home messages
 Present in 3 ways when unwell
 CCF – L to R shunt
 Cyanotic – R to L shunt
 Shock/collapse - LVOTO
 Key Sx/Sn
 DDx for the wheezing infant
 Always check the liver and femorals
 Prostaglandin E 1 can be life saving
 Get help early if CHD is suspected